Buso, Helena, Firing, Davide, Gambier, Renato, Scarpa, Riccardo, Garzi, Gilulia, Costanzo, Giulia, Ledda, Andrea G., Rashidy, Nicolo, Bertozzi, Ilaria, Nicola, Stefania, Tessarin, Giulio, Ramigni, Mauro, Piovesan, Cinzia, Vianello, Fabrizio, Jones, Mark G., Agostini, Carlo, Rattazzi, Marcello and Cinetto, Francesco (2024) Lung function trajectories in common variable immunodeficiencies: an observational retrospective multicenter study. Journal of Allergy and Clinical Immunology. (doi:10.1016/j.jaci.2024.10.037).
Abstract
Background: respiratory disease is a frequent cause of morbidity and mortality in Common Variable Immunodeficiencies (CVIDs), however lung function trajectories are poorly understood.
Objective: to determine lung physiology measurements in CVID, their temporal trajectory and association with clinical and immunological parameters.
Methods: retrospective study from 5 Italian centres. CVIDs patients with longitudinal Pulmonary Function Tests (PFTs) measurements and available chest CT scan, were included. Applying the ERS/ATS 2021 standard, PFTs were expressed as percentile value (pct) within the normal distribution of healthy individuals, with the 5th pct identified the lower limit of normal (LLN). The association of lung function with clinical and immunological parameters was investigated.
Results: 185 CVIDs patients were included. 64% had at least one lung comorbidity (bronchiectasis 41% and Granulomatous Interstitial Lung Diseases 24%). At first spirometry, the median FEV1 was 3.07 L [IQR 2.40 - 3.80], placing at the 32nd pct [6th-61st], and the median FVC was 3.70 L [3.00 - 4.54], placing at the 29th pct [7th-49th]. 23% of patients had an FEV1 < LLN and 15% had an FVC < LLN. Switched-memory B cells <2% were associated with both FEV1<LLN (OR 7.58) and FVC<LLN (OR 3.55). In 112 patients with at least 5 years of PFT follow-up, we found no significant difference between measured and predicted annual decline of FEV1 (25.6 vs 20.7 mL/year) and FVC (15.6 vs 16.2 mL/year).
Conclusion: our findings suggest that lung volumes of the majority of CVID patients placed in the lower third of normal distribution of healthy individuals. After diagnosis the rate of lung decline was not accelerated.
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